Cure Alveolar Soft Part Sarcoma International (iCureASPS)

We just recently found that Ivan now got 2 of them in one of his adrenal glands - last weekend on Aug.11, 2013 = almost 10 years after his initial Dx in Nov.2003. He arranged the abdominal CT scan with the contrast by his own initiative feeling that after his brain met pooped up from nowhere he needs to be more vigilant - if you pass to the next level of this survivor game the difficulty level increases.
As far as I know this is the first documented case of the ASPS adrenal met. Apparently the adrenal glands are the organ very frequently affected by the mets in a few diseases one of them is RCC, but they are very often missed as they are completely asymptomatic when small.
Fortunately this time the radiologist did his best and did not miss it! The sizes are kind of small - about 10-12 mm and Dr.Littrup (interventional radiologist cryoablation genius) is confident that he can ablate them reliably completely with the minimal probability of any complications. We are hoping that he would be able to save some piece of the gland - it is really small organ attached to a kidney on top. The contra-lateral gland is able to fulfill the production of the hormones fully but it is very desirable to have two working gland just in case. Apparently it only needs to have a small piece of the tissue to remain healthy as it has an ability to hypertrophy and became bigger after it heals. This is a part of the reason we are flying tomorrow to Detroit and paying our own money to have it done by Dr.Littrup instead of pursuing the local Dr. to do that paid by the provincial health insurance. our local doc is also a very good one but we are hoping that Dr.Littrup's extremely high level of the specific skills and the best available cryo units that exist now would give us one more chance to save the gland and get trough this problem with the minimal complications.
The procedure is planned on an outpatient basis, with only few days in Detroit if everything goes well. We are staying at the Holiday Inn Express downtown and are expected to arrive to DMC Tuesday morning for the procedure. I will keep everyone updated.

Dear Olga,
Thank you so much for so graciously sharing this information in the midst of your emergency scheduling of Ivan's Cryoablation, impromptu making of all of the necessary travel arrangements, and having to so quickly get ready to fly to Detroit. I am so very sorry for the diagnosis of Ivan's two adrenal gland mets, but am deeply grateful that Cryoablation is a viable treatment option and that arrangements were able to be so quickly made for him to have the procedure done with Dr. Littrup today. He and you are being held very close in all of us Hess's hearts and thoughts, and we are holding very tight to Hope for a very successful outcome to the procedure and a speedy and full recovery for Ivan with part of his adrenal gland having been able to be saved and remain functional.
Ivan's rare diagnosis of ASPS adrenal gland mets is a harsh reminder to all of us in the ASPS Community that this insidious and unpreditable disease can, and sometimes does, metastasize almost anywhere in the body as happened to Brittany when she was diagnosed with the very rare ASPS pancreatic met in the head of her pancreas which devastatingly was not resectable nor ablatable. Our only treatment option for the Life threatening pancreatic met was a systemic treatment, and thankfully Cediranib was successful in shrinking and ultimately destroying the tumor.
I Hope that everyone on this Board will learn from Ivan's and Brittany's experiences about how extremely important vigilant and complete scanning is to find and treat any possible new mets at their smallest and most treatable sizes, and BEFORE they become symptomatic which may then tragically be too late for resection, ablation, or radiosurgery.
I will be anxiously awaiting your or Ivan's update on the results of his Cryoablation when your time and the situation allow.
Take care dear Olga and feel the special embrace of my deepest caring, very best wishes, warm friendship, and continued Hope,
Bonni

The ablation was done by Dr.Littrup on Aug.20,2013. It was done under the deep sedation (not the general anaestesia) which is beneficial for the patient general health. It went good, the ablation itself was easy as Dr.Littrup said but there was a spike in the blood pressure during the thaw phase of the ablation cycle. They were aware of this possible occurring from their previous experience with the adrenal ablutions and inserted the additional port arterial port that needed to be stitched in - so the parathion took more time than usual. So when the blood pressure spiked - shoot thought the roof till 300 - they were ready and gave him the med right away and it worked. Aside of that he had lots of nausea and vomiting as a possible side effect front the sedation meds and it took more than 12 hours to unwind. We checked in at 8 am and left the hospital at 5-30 pm so it was an outpatient procedure, went to our Holiday Inn room and stayed there for the rest of the evening unable to go anywhere as Ivan was not well with all the nausea and vomiting. Woke up the next day feeling better. Dr.Littrup said that he tried to save as much adrenal tissue as possible but it remains to be seen if what is left will hyper trophy after it heals.

Some important note about the scanning. The initial scan that found these adrenal mets was a CT with the contrast. We had a previous scans with contrast to compare to. It would not have been found on a CT without a contrast. By some reason the radiologist did not compare the scan to the lung ct with the contrast that Ivan had before where these nodules can already be seen as the upper portion of the kidneys is located in the chest field nd the interval growth is evident. Not knowing that the radiologist recommended to wait till the next scan, rescan to see and verify the growth. Had we followed this recommendation, the consequences might have been tragic as from that comparacement Ivan done himself we knew that these mets grow rather fast about 2 mm per month. The oncologist decided to additionally scan Ivan by the PET/CT trying to make sure that theses nodules are mets versus some benign growth - apparently the benign adrenal nodules are not rare. I suggested that MRI would be a better choice, but the availability of this scan is poor in Canada and she went with the PET/CT ahead as she could arrange it very soon - the same week. The PET scan was not at all helpful in ruling out the possible adrenal mets - it did not see anything, these mets did not lot up on the scan tall which we now know is a truly false negative result - Dr. Littrup measured during the procedure and confirmed that the nodules have grown additionally by more than 2 mm in less than a month since the scan, this speed of growth is incompatible with the any benign nodules that might be found in the adrenal glands. So in fact the results of the Pet/CT would have been misleading if we didn't know that Pet has very low sensitivity for the smaller ASPS tumors.

Dr.littrup has recommended to follow-up these nodules by the MRI scan in 1-3 months to make sure the ablation is complete.

Dear Olga,
Thank you for your thoughtful update and all of the very important shared information. I am so sorry that Ivan suffered so much post-Cryo nausea and vomiting, but Hope that he is now feeling much better and recovering well. I assume that the nausea and vomiting were related to the increased dose of sedation medication and possibly the fact that his procedure was done on his abdominal area which is probably reacting to the intrusion in that area and the freezing/destruction of tissue in the adrenal gland. It is very frightening that Ivan's blood pressure spiked so high during the procedure, but I am so very grateful for Dr. Littrup's experience, expertise, and the preventative measure he had taken to keep the situation under control and quickly resolve it. Although I know that it is a significant increased financial and logistic hardship to have the procedure done out of the country in the USA instead of locally in Canada, I think that you made a very good and wise choice in choosing to have the Cryo done with Dr. Littrup.
The critically important scanning information which you have so graciously shared should be of greatest interest and utmost importance to everyone in the ASPS Community including the doctors, patients, and family members and I Hope that they will all learn and benefit from your experience and the information that you have provided. As both your family and our family have learned through our combined 22 years of shared ASPS experiences, vigilant, adequate, and appropriate scanning is essential in managing and fighting this very challenging disease combined with pro-actively and aggressively pursuing the best and most appropriate treatment of any new mets as soon as possible based on up-to-date research and anecdotal treatment knowledge.
Take care dear Olga, give yourself and Ivan gentle hugs from all of us Hesses, travel safe back to Vancouver, and keep the Board updated on Ivan's recovery and ablation results as you are able.
With special caring thoughts, healing wishes for Ivan, and continued Hope,
Bonni

Olga and Ivan

We are so glad that this part of the journey is over with and you've managed it with such grace and control
What kinds of tests will you be up against to check function of the glands? Would it be possible for a doctor to feel these mets during an exam for other patients now that we know the gland is a possibility ?

So you will be scanning your lower abdomen in 3 months?
Did you have any sweating or other symptoms with these mets or do you think they were still too small and non symptom producing ?
Being that this post is about the adrenal I will close with
Thank you both for your answers.

Love
Debbie

All the good questions, Debbie.
1.
Q. will you be up against to check function of the glands?
A. Adrenal insufficiency is checked by the blood test. We will ask the family doc. to get the test done. The usual outcome of the one side adrenalectomy is the complete restoration of the balance by the way of the other side gland hypertrophy and increasing production to work for two.

2.
Q. Would it be possible for a doctor to feel these mets during an exam for other patients now that we know the gland is a possibility ?
A.I do not think so. From what I saw on the scan, It as located inside between the kidney and spinal column. There are numerous reports of the cases on the Pubmed when adrenal mets from other faster growing diseases were found by the bulk effect they produced pressing on the other organs, but this is a very suboptimal way of finding the met. The best way to find and treat a met is when it is asymptomatic - it means that it does not press on or grows into anything - treating the met like that is much easier.

3.
Q. So you will be scanning your lower abdomen in 3 months?
A. Dr.Littrup gave us a letter for the oncologist suggesting the follow up scanning in 1-3 months using MRI (not CT with the contrast) because it is a good high sensitivity ans specificity scan for non-moving organs and does not use radiation to lower down the dose Ivan gets from the scanning. We are going to discuss with the oncologist the possibility of having abdominal MRI versus CT scans overall as a periodic abdomen surveillance scan.
I do not know if it is going to be seen on the first after ablation scan if any of the treated gland tissue survived the procedure. I will ask that the scan would be reviewed by the kidney doctor in addition to the radiologist.

It is almost 3 years since Ivan was first Dx with the adrenal mets. It was cryoablated by Dr.Littrup in attempt to save the adrenal function. After that he had a local satellite recurrence on the same side a year later, between the adrenal and the Inferior vena cava (IVC). It was suggested that the full size abdominal surgery with the IVC reconstruction is the only chance to deal with it, but Dr.Littrup based on the work previously conducted that the cryo close to IVC allows for the very deep freeze as the heat sink caused by the fast hot blood flow is not only an enemy but also a friend here - it protects the IVC from breaking. So he cryo-ed that area again. Two years after there is no recurrence there. But Ivan had the new adrenal met a year and a half ago in the contralateral adrenal - left one. We were pretty glad that the first one affected - left one - was not surgically removed and there is still a chance there is some remaining tissue that keeps working now. So we went for the right adrenal cryo again as the location was favorable with the excentric location, allowing to cryoablate half and to preserve the other half. One year and 1 month ago it was done, and now there is no local recurrence (found in July 2016). But we have the satellite recurrence again in the left side close to adrenal again. It is either between the adrenal and the pancreas or arises from the out payer of pancreas in its middle- close to the tail area. Unfortunately the complete cryoablation or other types of ablation in this area are not possible without the great damage to the pancreas duct. So we are now looking for the minimally invasive surgery option to remove it.